Circulation, Vol 85, 116-122, Copyright © 1992 by American Heart Association
PE Farrell Jr, AC Chang, KA Murdison, JM Baffa, WI Norwood and JD Murphy
BACKGROUND. We reviewed the outcome of 76 consecutive patients (age range,
5 months to 6 years; median age, 19 months) who underwent a modified Fontan
procedure after initial palliative surgery for hypoplastic left heart
syndrome (HLHS) between January 1984 and December 1989. METHODS AND
RESULTS. Modifications of the Fontan procedure included transatrial baffle
of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena
cava baffle within the right atrium to the superior vena caval-pulmonary
artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial
survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52%
(4 years). Of the 43 survivors, 25 patients have returned for postoperative
cardiac catheterization at a medium of 13 months after the Fontan
procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6
l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge
pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No
patient had significant tricuspid or native pulmonary valve insufficiency.
CONCLUSIONS. Survival after the Fontan procedure in patients with HLHS is
comparable to survival after a Fontan procedure in patients with other
complex congenital heart lesions. In the subgroup of patients with HLHS who
survived both reconstructive surgery and a Fontan procedure and have been
evaluated by cardiac catheterization after a Fontan procedure, the use of
the right ventricle as the systemic ventricle yielded excellent
intermediate results for Fontan physiology.
ARTICLES
Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome
Division of Cardiology, Children's Hospital of Philadelphia, PA 19104.
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